{"product_id":"abcam-ab152216","title":"Abcam, ab152216, Recombinant Human ATP7b protein","description":"\u003cp\u003eSize: 10µg\u003cbr\u003e\nRecombinant Human ATP7b protein is a Human Fragment protein, in the 1372 to 1465 aa range, expressed in Wheat germ and suitable for western blot, SDS-PAGE and ELISA. The predicted molecular weight of ab152216 protein is 36 kDa. - Save time and ensure accurate results - use our ATP7b protein as a control\u003cbr\u003e\nKey facts\u003cbr\u003e\nExpression system:Wheat germ,\u003cbr\u003e\nTags:GST tag N-Terminus,\u003cbr\u003e\nApplications:SDS-PAGE, ELISA, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nBiologically active:No,\u003cbr\u003e\nAccession:P35670,\u003cbr\u003e\nAnimal free:No,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nSpecies:Human,\u003cbr\u003e\nStorage buffer:pH: 8Constituents: 0.79% Tris HCl, 0.31% Glutathione\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nEnsure the validity of your result using our recombinant human ATP7b protein ab152216 as a positive control in western blot and SDS-PAGE.\u003cbr\u003e\nAnalyze your ATP7b ELISA data using the ab152216 protein to generate and plot a standard curve.\u003cbr\u003e\nCheck out our protein gel staining guide for SDS-PAGE\u003cbr\u003e\nCheck out of western blot protocol for more information\u003cbr\u003e\nCheck out our ELISA protocol for more information\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Dry Ice, Appropriate short-term storage conditions--80°C, Appropriate long-term storage conditions--80°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nATP7B also known as Wilson disease protein is a copper-transporting ATPase. This protein has a mass of approximately 146 kDa. It is mainly expressed in the liver kidney placenta and brain. ATP7B functions mechanically by transporting copper across different cellular compartments. It utilizes ATP hydrolysis to pump copper ions helping the body regulate copper homeostasis. This transport activity is located predominantly in the trans-Golgi network where ATP7B assists in incorporating copper into ceruloplasmin a critical copper-carrying blood plasma protein.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nATP7B plays a significant role in maintaining copper balance within the body. It associates with intracellular vesicles and through its catalytic activity influences various cellular metabolic processes. Although ATP7B does not typically form large complexes its interaction with other proteins and cellular organelles contributes to copper ion binding and transfer. Proper ATP7B function ensures that copper is channeled effectively to places where it is required for enzymatic activity or is expelled from cells to prevent accumulation.\u003cbr\u003e\nPathways\u003cbr\u003e\nATP7B is a central figure in the copper transport and homeostasis pathway. Its role is directly connected to the biosynthesis of copper-dependent enzymes. The protein impacts the pathway involving ceruloplasmin biosynthesis by regulating copper ion incorporation. ATP7A another copper-transporting ATPase shares similar pathway responsibilities but in different tissues showing divergence in their specific biological roles. Together they ensure whole-body copper balance.\u003cbr\u003e\nATP7B mutations or dysfunction is strongly implicated in Wilson's disease a genetic disorder characterized by excessive copper accumulation. This can result in hepatic neurological and psychiatric symptoms due to copper buildup. The protein's relationship to Wilson's disease suggests its critical role in preventing copper toxicity. There is also evidence linking ATP7B with Menkes disease but ATP7A plays a more direct role in Menkes highlighting the distinct responsibilities these ATPases have in different tissues and conditions.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46843892662441,"sku":"ab152216","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab152216","provider":"Iright","version":"1.0","type":"link"}